Haematology p186 Flashcards

1
Q

components of blood

A

plasma 55%

red blood cells 44%

WBCs, platelets 1%

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2
Q

plasma components

A

7-9% proteins

90% water

  1. 1% sugar
  2. 03% urea
  3. 9% salt
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3
Q

red blood cells

A

carry oxygen and Hb

release ATP which cause vessel walls to relax to promote blood flow

when lysed pathogenically by bacteria the Hb in the RBC produces free radicals which break down the bacterial cell membrane destroying it

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4
Q

anaemia is

A

reduction of Hb in blood (not necessarily red cells)

  • red cell reduction is indicative of marrow failure

anaemic pt have below average Hb

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5
Q

av male Hb

A

13.5-17.5g/dl

anaemia male - <13g/dl

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6
Q

av female Hb

A

12.0-15.5d/dl

anamic female 11.5g/dl

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7
Q

general clinical features of anamia

A

hypoxia - compensatory changes = inc HR + SV = CO

pallor - due to vascular constriction in skin

fatigue

tachycardia (to try and make up for poor oxygen transport)

may develop a murmur

in elderly it can manifest as palpitations, dyspnoea, angina and signs of caridac failure

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8
Q

histological classifications of anaemia

A

hypochromic microcytic (pale small cells)

  • iron def, thalassaemia, some chronic disease

normochromic normocytic (normal colour, normal size)

  • chronic disease (heart failure, renal failure), acute blood loss

macrocytic (large cells)

  • megaloblastic (B12, folate), aplastic, liver disease
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9
Q

classes of anaemia

A

haematinic deficiency anaemias

haemaglobinopathy anaemias (globin chains)

marrow failure anaemias (reduced RBC)

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10
Q

3 marrow failure anaemias

A

aplastic anaemia

acute leukaemia (neoplastic proliferation of leukocytes, usually disseminated)

lymphoma (neoplastic proliferation of leuokcytes, usually solid tumour)

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11
Q

2 haemoglobinopathy anaemias

A

thalassaemia

sickle cell anaemia

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12
Q

haematinic deficiency anaemia (2)

A

iron deficiency anaemia

perncious (B12) megaloblastic (B9/folate) anaemias (def B12 and folic acid)

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13
Q

iron def anaemia

A

Causes include chronic blood loss, malabsorption of iron (coeliac disease), dietary deficiency, increased physiological demand (e.g pregnancy, puberty), Low ferritin (stores and releases iron)

Clinical features include

  • Kolinychia (concave nails)
  • Pruritus (itching)
  • Angular Stomatitis
  • Painless glossitis (beefy tongue)

Low Hb, Low MCV + MCH

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14
Q

pernicious megaloblastic anaemias

A

def B12 and folic acid

Both B12 and Folic acid needed for DNA synthesis in maturing erythroblasts

  • Def. in either causes erythroblasts to have large nuclei= megaloblasts in marrow

B12 absorbed by intrinsic factor in terminal ileum

Folate absorbed in the duodenum

Clinical features include

  • Painful glossitis
  • Demyelination of the spinal cord
  • Peripheral neuropathy
  • Ataxia

Low Hb, High MCV

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15
Q

Thalassaemia

A

Normal Hb production

Genetic mutation of globin chains

  • α chains (Asian)
  • β chains (Mediterranean)

Clinical features include

  • Chronic Anaemia
  • Marrow Hyperplasia
  • Spenomegaly
  • Cirrhosis
  • Gallstones

Treated w/ blood transfusions
Prevent iron overload

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16
Q

sickle cell anaemia

A

β chain substitution

Changes shape in hypoxic environment (prevents passage of cells through capillaries)

Heterozygous- Sickle cell TRAIT
Homozygous- Sickle Cell DISEASE

Clinical features include

  • Vascular Occlusion
  • Retinal Ischaemia
  • Acute chest syndrome
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17
Q

aplastic anaemia

A

Diminished or absent Haematopoetic precursors in bone marrow

Present as, Pancytopenia, Macrocytosis (inc. MCV), reticulocytopenia (immature RBCs)

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18
Q

acute leukaemia

A

Accumulation of malignant white cells in bone marrow and blood

Malignant cells tend to be precursors (blast cells)

Two types Acute MYELOID leukamia and Acute LYMPHOBLASTIC leuakaemia

Neutropenic- ROU

Thrombocytopenia- Mucosal Bruising, Petechiae, epistaxis (nosebleed) and Gingival Hypertrophy

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19
Q

lymphoma

A

Malignant lymphocytes accumulating in Lymph nodes

  • Lymphadenopathy, Peripheral vasculature, even organs

Nodular Lymphocyte-predominant Hodgkin’s Lymphoma (NLPHL) - malignant B cells lying in a meshwork of folicular dendritic cells and reactive lymphocytes

Classical Hodgkin’s Lymphoma- neoplastic B cells amongst Reed-Sternberg (RS) cells and plasma cells + eosinophils

Presence of RS cells determines whether it is a Hodgkin’s or Non-Hodgkin’s Lymphoma

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20
Q

normal RCC

male

A

4.7-6.1 million cells/mcL

usually normal in anaemia

check if normochromic/cytic

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21
Q

normal RCC

females

A

4.2-5.4 million cells/mcL

usually normal in anaemia

check if normochromic/cytic

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22
Q

WCC normal

A

between 4.5 and 10.0 thousand cells/mcL

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23
Q

MCV is

A

mean corpuscular volume (volume RBC’s take up, a.k.a size)

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24
Q

normal MCV

A

87 +/- 5 femtolitres (fL)

will be normal in bleeding but reduced volume overall

25
Q

HCT is

A

haematocrite

% vol of RBC in blood

26
Q

HCT in newborns

A

60%

0.6

varies in children

27
Q

HCT in males

A

40-52%

0.4-0.52

28
Q

HCT in females

A

46%

0.46

can be reduced in pregnancy

29
Q

PLT is

A

platelets

30
Q

normal PLT

A

150-400 thousand per mcL

31
Q

ferritin in males

A

18-270mcg/L

32
Q

ferritin in females

A

18-160mcg/L

33
Q

polycythaemia

A

raised Hb

34
Q

leukocytosis

A

raised WCC

35
Q

thrombocythaemia

A

raised PLT

36
Q

reasons for needing a blood transfusions

A

When one or more components of the blood has to be replaced quickly, i.e in blood loss

  • e.g RBCs, PLTs, Clotting factors

When bone marrow can’t produce blood cells

  • e.g RBCs, PLTs
37
Q

if have only just one odd number in bloods

if multiple

A

reactive change?

more likely pre neoplastic - investigate

38
Q

how to blood transfuse

A

Sample taken from patient

  • ABO compatability
  • Rhesus +ve or -ve
  • Sometimes there are unknown antigens present

Tested v donated sample, if matched = given to patient

If poor match- fever, jaundice, fluid overload

Prion Disease- neurogenerative conditions

Bacteria (Syphilis) and Viruses (Hep B, C, HIV and TT viruses) can all be transmitted via transfusion of blood

39
Q

porphirins are

A

proteins involved in Hb metabolism

40
Q

2 main types of porphyria

A

hepatic porphyria

erythropoetic porphyria

41
Q

acute episodes of porphyria cause

A

purple urine, abdominal pain, seizures, skin rash from sunlight and psychosis

42
Q

clotting factor VII def is

A

haemophilia A

X chromosome recessive inherited bleeding disorder (higher male incidence)

43
Q

clotting factor IX deficiency is

A

haemophillia B (christmas disease)

mutaration of FIX gene on the X chromosome

results in low/absent level of FIX gene in plasma

44
Q

severtity of haemophilia A correlates to

A

level of Factor VIII in blood

45
Q

DDAVP is

A

desmopressin

can be usde to tx mild haemophilia and vWD by stimulating release of vWF

46
Q

Von Williebrand’s disease

A

vWD

decrease in von Williebrand’s factor which causes a reduced factor VIII level

vWF acts as a carrier molecule for Factor VIII hence why it can’t be degraded to be used in coagulation as it isn’t near the site of insult

reduced platelet aggregation

47
Q

3 acquired bleeding disorders

A

disseminated intravascular coagulation DIC

vit K deficiency

hepatopathology

48
Q

disseminated intravascular coagulation

A

Consumption of clotting factors widespread

Causes microthrombi to form

excess consumption of factors and platelets means intial coagulopathy then excess bleeding

49
Q

vit k def due to

A

malabsorption in GIT

50
Q

hepatopathology causes

A

reduced level of clottisng factors produced so bleeding disorder

51
Q

acquired platelet disorders

A

Thrombocytopaenic disorders

  • Acute Idiopathic Purpura (Children)
    • following a viral infection
    • autoantibodies bind to platelets and cause them to be removed from circulation and destroyed by spleen
  • Myeloproliferative disorders
    • Bone marrow doesn’t produce enough platelets causing systemic low platelet count

Acquired disorders of platelet function

  • Antiplatelet agents
52
Q

antiplatlet agents

A

Aspirin inhibits Cyclo-oxygenase enzyme which is needed to synthesise thromboxane a2 which is needed to activate platelets

Heparin

Penicillins

53
Q

hypercoagulation called

A

thrombophilia

54
Q

heriditary thrombophilia

A

factor V Leiden mutation

protein C

protein S

anti-thrombin III def

55
Q

acquired thrombophilia

A

antiphospholipid syndrome (Hughs syndrome)

caused by presence of Lupus anti-coagulant in blood

sugery, trauma, immobilisation, cancer etc

56
Q

increased platelets called

A

thrombocythaemia

57
Q

essential throbocytosis

A

inc platelets

v uncommon

managed with aspirin

58
Q

INR is

A

internal normalised ratio

prothrombin time

Ratio of patients clotting value over the normal clotting value

Normal INR- = 1.0
Warfarin INR- 2.0-3.0
-

people who have AF or leg/lung clots have higher INR ratio

LOWER THE INR THE FASTER THE CLOTTING TIME